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Pentalogy of Cantrell with encephalocele – A case report with review of literature

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Case Report

Author Details : Suriya Desikan*, Sendhil Coumary A, Syed Habeebullah

Volume : 8, Issue : 2, Year : 2021

Article Page : 275-278

https://doi.org/10.18231/j.ijogr.2021.058

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Abstract

Pentalogy of Cantrell is a rare structural disorder. It is a combination of congenital defects in the midline comprising of supraumbilical abdominal wall defect, defect in the lower sternum, deficient anterior diaphragm, defect in diaphragmatic pericardium and intracardiac defects. It is proposed to result from an insult occurring as early as 14th to 17th day of embryonic life. It involves the ventral midline due to disruption of midline mesenchyme. Etio-pathogenesis is still largely unknown. The insult could be environmental or disruption of a single gene, either autosomal or X linked. Very rarely Cantrell’s syndrome is associated with neural tube disorders. Here, we report one such rare case of Pentalogy of Cantrell.

Keywords: Pentalogy of Cantrell, Encephalocele, Ectopia cordis.

 

How to cite : Desikan S , Sendhil Coumary A, Habeebullah S , Pentalogy of Cantrell with encephalocele – A case report with review of literature. Indian J Obstet Gynecol Res 2021;8(2):275-278

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